Uveal melanoma: natural history and treatment options for metastatic disease

In order to evaluate the natural history, prognostic parameters and treatme nt modalities for metastatic uveal melanoma, a review of the clinical data from the current literature was performed based on a Medline database searc h. Uveal melanoma represents approximately 5% of all melanomas. It is a dis tinct clinico-pathological entity, differing in many aspects from cutaneous melanoma. The clinical course is unpredictable and metastatic disease can develop very late after a long disease-free interval. Uveal melanoma metast asizes haematogenously, predominantly to the liver. The most important prog nostic parameters for primary uveal melanoma are tumour diameter, the patie nt's age and gender, histological features and tumour location. Systemic ch emotherapy that is effective in cutaneous melanoma has failed to show activ ity in uveal melanoma. So far only the BOLD chemotherapy regimen (dacarbazi ne, lomustine, vincristine and bleomycin) combined with interferon-alpha ha s been shown to produce an objective tumour response in approximately 20% o f previously untreated patients. For metastatic disease localized to the li ver, intraarterial application of fotemustine or carboplatin or chemoemboli zation with cisplatin have shown useful activity, resulting in a response i n up to 40% of patients. Selected patients may benefit from palliative surg ery. Immunotherapy with interleukin-2 or interferon-alpha has not shown con sistent activity in metastatic uveal melanoma. In conclusion, patients with uveal melanoma metastatic to the liver should undergo one of the local tre atment options. Care fully selected patients with extrahepatic disease or p atients failing local treatment may benefit from systemic therapy using the BOLD regimen combined with interferon.